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Zhou Y, Chen X, Li J, Luo F. Ruptured pancreatic carcinosarcoma - A case of extended survival following surgical resection and chemotherapy. REVISTA ESPANOLA DE ENFERMEDADES DIGESTIVAS 2024. [PMID: 39530578 DOI: 10.17235/reed.2024.10856/2024] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/16/2024]
Abstract
We present a rare case of ruptured pancreatic carcinosarcoma in a 56-year-old male who underwent radical surgery and adjuvant chemotherapy. Despite the typical poor prognosis associated with this aggressive tumor, the patient achieved an overall survival of 20 months postoperatively. This case emphasizes the challenges in diagnosing and managing pancreatic carcinosarcoma and highlights the potential benefits of aggressive surgical and chemotherapeutic interventions in achieving extended survival.
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Affiliation(s)
- Yekai Zhou
- Hepatobiliary Surgery, The First Affiliated Hospital of Chongqing Medical University , China
| | - Xiaorong Chen
- General Surgery, Jiangbei Campus, The First Affiliated Hospital of Army Medical University
| | - Jie Li
- Hepatobiliary Surgery, The First Affiliated Hospital of Chongqing Medical University
| | - Fang Luo
- Hepatobiliary Surgery, The First Affiliated Hospital of Chongqing Medical University
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2
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Fleming AM, Hendrick LE, Glazer ES, Murphy AJ, Dickson PV, Shibata D, Yakoub D, Deneve JL. Pancreatic carcinosarcoma: An orthogonal analysis. Surgery 2023; 174:907-915. [PMID: 37524640 DOI: 10.1016/j.surg.2023.06.039] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/14/2023] [Revised: 05/10/2023] [Accepted: 06/18/2023] [Indexed: 08/02/2023]
Abstract
BACKGROUND Pancreatic carcinosarcoma is a rare subtype of pancreatic cancer. There are no consensus guidelines regarding its treatment. The current study is an orthogonal analysis of multiple datasets to evaluate prognostic features. METHODS A modified Preferred Reporting Items for Systematic reviews and Meta-Analyses 2020 systematic review was performed for reported cases of pancreatic carcinosarcoma. All cases of pancreatic carcinosarcoma in the National Cancer Database were identified for analysis. Analyses were compared to previously published data from the Surveillance, Epidemiology, and End Results database to increase validity. RESULTS Seventy-one cases of pancreatic carcinosarcoma were reported in the literature. Reports of pancreatic carcinosarcoma increased over time (P = .0075). Tumor size >5.0 cm, metastatic disease, and relapse were associated with decreased disease-specific survival (all log-rank P < .05). Ninety-nine cases of pancreatic carcinosarcoma were analyzed within the National Cancer Database. Pancreatic carcinosarcoma incidence increased over time (P = .0371). Resection + chemotherapy, pathologic lymph node examination, and treatment at an academic center were associated with improved overall survival (all log-rank P < .05), whereas harboring ≥2 positive lymph nodes was associated with decreased overall survival (log-rank P = .0171). Within a multivariable model adjusting for age, sex, comorbid disease, and disease stage, resection + chemotherapy was associated with a decreased hazard of death (hazard ratio .036; confidence Interval .004-.298; P = .0022). Published data from the Surveillance, Epidemiology, and End Results database supported the current analysis regarding the incidence of pancreatic carcinosarcoma, resection, lymph node evaluation, and the impact of metastatic disease. CONCLUSION Pancreatic carcinosarcoma is exceedingly rare, with a poor prognosis. Long-term survival is possible with curative resection in the absence of relapse. The number of positive lymph nodes appears to impact prognosis.
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Affiliation(s)
- Andrew M Fleming
- Department of Surgery, The University of Tennessee Health Science Center, Memphis, TN; Department of Surgery, St. Jude Children's Research Hospital, Memphis, TN.
| | - Leah E Hendrick
- Department of Surgery, The University of Tennessee Health Science Center, Memphis, TN. https://twitter.com/LeahHendrick8
| | - Evan S Glazer
- Department of Surgery, The University of Tennessee Health Science Center, Memphis, TN. https://twitter.com/EvanGlazerMD
| | - Andrew J Murphy
- Department of Surgery, The University of Tennessee Health Science Center, Memphis, TN; Department of Surgery, St. Jude Children's Research Hospital, Memphis, TN. https://twitter.com/ajacksonmurphy
| | - Paxton V Dickson
- Department of Surgery, The University of Tennessee Health Science Center, Memphis, TN
| | - David Shibata
- Department of Surgery, The University of Tennessee Health Science Center, Memphis, TN
| | - Danny Yakoub
- Division of Surgical Oncology, Augusta University Medical Center, Augusta, GA
| | - Jeremiah L Deneve
- Department of Surgery, The University of Tennessee Health Science Center, Memphis, TN. https://twitter.com/jigdeneve
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Khan J, Cheng L, House MG, Guo S. Carcinosarcoma, a Rare Malignant Neoplasm of the Pancreas. Curr Oncol 2021; 28:5295-5303. [PMID: 34940081 PMCID: PMC8699933 DOI: 10.3390/curroncol28060442] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/12/2021] [Revised: 12/08/2021] [Accepted: 12/10/2021] [Indexed: 11/16/2022] Open
Abstract
Carcinosarcoma of the pancreas is a rare entity with poor prognosis. Here, we report a case of pancreatic carcinosarcoma in a 68-year-old male patient who underwent a pancreatoduodenectomy for a unilocular cystic mass in the head of the pancreas. Histologically, the lesion showed a biphasic tumor with a carcinoma component and a spindle cell sarcomatous component, which were intimately intermingled. Most of the carcinoma components are well-differentiated ductal adenocarcinoma with small areas of moderately to poorly differentiated ductal adenocarcinoma. The sarcomatous component is a high-grade highly cellular spindle cell tumor with frequent mitosis and apoptosis. Immunohistochemical studies demonstrated that the carcinomatous component was positive for epithelial markers and cyclin D1, and the sarcomatous component was negative for these markers while positive for vimentin, p16, and DOG1 with patchy positivity for S100. Other markers, including SOX10, CD117, Melan A, HMB45, actin, desmin, myogenin, beta-catenin, TLE1, and p53, were negative in both components. Molecular studies demonstrated that the tumor was microsatellite stable. Whole exome next generation sequencing analysis was performed and no pathogenic alterations in the genes were identified.
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Affiliation(s)
- Jaffar Khan
- Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN 46202, USA; (J.K.); (L.C.)
| | - Liang Cheng
- Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN 46202, USA; (J.K.); (L.C.)
| | - Michael G. House
- Department of Surgery, Indiana University School of Medicine, Indianapolis, IN 46202, USA;
| | - Shunhua Guo
- Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN 46202, USA; (J.K.); (L.C.)
- Correspondence:
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Lim HJ, Kang HS, Lee JE, Min JH, Shin KS, You SK, Kim KH. Sarcomatoid carcinoma of the pancreas — multimodality imaging findings with serial imaging follow-up: A case report and review of literature. World J Clin Cases 2021; 9:3102-3113. [PMID: 33969097 PMCID: PMC8080743 DOI: 10.12998/wjcc.v9.i13.3102] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/12/2020] [Revised: 01/28/2021] [Accepted: 03/04/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Sarcomatoid carcinoma of the pancreas is extremely rare and has an extremely poor prognosis. Although a few cases of sarcomatoid carcinoma of pancreas have been reported, most are focused on a histopathological review. To the best of our knowledge, there are no reports documenting multimodality imaging characteristics and chronological changes with emphasis on radiologic features.
CASE SUMMARY A 64-year-old woman was admitted to Chungnam National University Hospital with acute appendicitis. Contrast-enhanced computed tomography of the abdomen revealed a 2.6 cm × 2.8 cm multilobular cystic mass in the pancreatic tail. The pancreatic lesion showed suspected mural nodules and thin septa. Hence, mucinous cystic neoplasm of pancreas was considered. After 7 mo, the patient was readmitted for repeated epigastric abdominal pain and nausea. Follow-up contrast-enhanced computed tomography of the abdomen and magnetic resonance imaging revealed a marked enlargement (5.4 cm × 4 cm), with a predominant internal solid component. The mass showed low signal intensity on a T1-weighted image and heterogeneously intermediate high signal intensity on a T2-weighted image. It showed diffusion restriction and peripheral rim enhancement on an arterial phase image, and progressive enhancement on portal venous and delayed phase images. Distal pancreatectomy was performed. Based on the morphology and immunohistochemical staining of the specimen, pancreatic sarcomatoid carcinoma was diagnosed.
CONCLUSION We present the computed tomography, magnetic resonance imaging, and positron emission tomography computed tomography findings, pathologic features, and chronological changes for preoperative diagnosis.
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Affiliation(s)
- Hyun Jae Lim
- Department of Radiology, Chungnam National University Hospital, Daejeon 35015, South Korea
| | - Hye Seon Kang
- Department of Radiology, Chungnam National University Hospital, Daejeon 35015, South Korea
| | - Jeong Eun Lee
- Department of Radiology, Chungnam National University Hospital, Chungnam National University College of Medicine, Daejeon 35015, South Korea
| | - Ji Hye Min
- Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 06351, South Korea
| | - Kyung Sook Shin
- Department of Radiology, Chungnam National University Hospital, Chungnam National University College of Medicine, Daejeon 35015, South Korea
| | - Sun Kyoung You
- Department of Radiology, Chungnam National University Hospital, Chungnam National University College of Medicine, Daejeon 35015, South Korea
| | - Kyung-Hee Kim
- Department of Pathology, Cancer Research Institute, College of Medicine, Chungnam National University, Daejeon 35015, South Korea
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Alhatem A, Quinn PL, Xia W, Chokshi RJ. Pancreatic Carcinosarcoma Clinical Outcome Analysis of the National Cancer Institute Database. J Surg Res 2020; 259:62-70. [PMID: 33279845 DOI: 10.1016/j.jss.2020.11.033] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/06/2020] [Revised: 10/19/2020] [Accepted: 11/01/2020] [Indexed: 12/12/2022]
Abstract
BACKGROUND Pancreatic carcinosarcomas (PCS) are rare aggressive biphasic malignancies with a poor prognosis. We aimed to improve the understanding of PCS by analyzing variables that influence the mortality of PCS patients. METHODS The Surveillance, Epidemiology, and End Results database was queried for cases of PCS from 1973 to 2016. Cases were analyzed for patient demographics, tumor characteristics, and surgical intervention. Kaplan-Meier and Cox regression analyses were applied to investigate the overall survival (OS) and prognostic factors. RESULTS Thirty-nine cases of PCS were identified along with the disease demographics and characteristics. The majority of patients had a regionally invasive or metastatic disease. There was a significant decrease in OS with the increase of the tumor extension. Conversely, surgery showed to improve OS in the crude analysis, including patients that underwent lymphadenectomy. In addition, the unadjusted Cox regression results showed decreased hazard ratios with a local disease versus distant metastasis and with cancer-directed surgery versus no surgery. Nevertheless, the adjusted Cox regression results revealed that metastatic disease was the only significant predictor of survival. CONCLUSIONS This population-based study provides some insight to a very rare disease by analyzing 39 cases of PCS. Our finding suggests considering PCS as a nonsurgical disease and reserving surgery solely for patients with a localized disease in combination or after neoadjuvant therapy. Consequently, there is a need to further investigate novel therapies for this aggressive malignancy.
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Affiliation(s)
- Albert Alhatem
- Rutgers New Jersey Medical School, Newark, New Jersey; Department of Pathology, Immunology and Laboratory Medicine, University Hospital, Newark, New Jersey
| | - Patrick L Quinn
- Rutgers New Jersey Medical School, Newark, New Jersey; Division of Surgical Oncology, Department of Surgery, University Hospital, Newark, New Jersey
| | - Weiyi Xia
- Biostatistics and Epidemiology Services Center, Rutgers School of Public Health, Rutgers University, Piscataway, New Jersey
| | - Ravi J Chokshi
- Rutgers New Jersey Medical School, Newark, New Jersey; Division of Surgical Oncology, Department of Surgery, University Hospital, Newark, New Jersey.
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Kimura T, Fujimoto D, Togawa T, Ishida M, Iida A, Sato Y, Goi T. Sarcomatoid carcinoma of the pancreas with rare long-term survival: a case report. World J Surg Oncol 2020; 18:105. [PMID: 32450860 PMCID: PMC7249341 DOI: 10.1186/s12957-020-01879-8] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/09/2020] [Accepted: 05/13/2020] [Indexed: 02/07/2023] Open
Abstract
BACKGROUND Sarcomatoid carcinoma of the pancreas (SCP) tends to have similar or even worse prognosis than that of conventional pancreatic ductal adenocarcinoma. The clinical and pathological features of SCP remain poorly characterized owing to its rarity. CASE PRESENTATION A 58-year-old man was admitted to our hospital with the chief complaints of upper abdominal pain and weight loss. Abdominal contrast computed tomography revealed a 5-cm low-density mass in the pancreatic body. Magnetic resonance cholangiopancreatography revealed an obstruction of the main pancreatic duct and a dilation of the distal main pancreatic duct. Based on the clinicoradiological findings, pancreatic body cancer was suspected, and the distal pancreatectomy was performed. A pathological examination revealed that the tumor was composed of an area of invasive ductal adenocarcinoma and an area of sarcomatous spindle-shaped cells; the latter component predominated. The spindle cells were immunohistochemically positive for both cytokeratin and vimentin, and thus, a pathological diagnosis of SCP was made. In addition, immunohistochemical analysis suggested the sarcomatous component might be derived from the adenocarcinoma component via the process of epithelial-mesenchymal transition. After the operation, the patient received 6 months of chemotherapy with gemcitabine. At 10 years after the operation, the patient is alive with no recurrence. CONCLUSIONS The current case study presented a SCP patient with long-term survival after the operation. It was worth noting that the sarcomatous component of the tumor pathologically showed lower MIB-1 labeling index compared with those in previously reported SCP cases, which might account for the long-term survival of the patient.
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Affiliation(s)
- Toshihisa Kimura
- grid.416698.4Department of Surgery, National Hospital Organization, Tsuruga Medical Center, 33-1, Sakuragaoka, Tsuruga, Fukui, 914-0195 Japan
| | - Daisuke Fujimoto
- grid.163577.10000 0001 0692 8246First Department of Surgery, Faculty of Medicine, University of Fukui, 23-3, Matsuoka Shimoaizuki, Eiheiji-cho, Yoshida-gun, Fukui, 910-1193 Japan
| | - Tamotsu Togawa
- grid.416698.4Department of Surgery, National Hospital Organization, Tsuruga Medical Center, 33-1, Sakuragaoka, Tsuruga, Fukui, 914-0195 Japan
| | - Makoto Ishida
- Department of Surgery, Tannan Regional Medical Center, 1-2-31, Saburoku-cho, Sabae, Fukui, 916-8515 Japan
| | - Atsushi Iida
- grid.416698.4Department of Surgery, National Hospital Organization, Tsuruga Medical Center, 33-1, Sakuragaoka, Tsuruga, Fukui, 914-0195 Japan
| | - Yasunori Sato
- grid.9707.90000 0001 2308 3329Department of Human Pathology, Kanazawa University Graduate School of Medicine, 13-1, Takara-machi, Kanazawa, Ishikawa 920-8640 Japan
| | - Takanori Goi
- grid.163577.10000 0001 0692 8246First Department of Surgery, Faculty of Medicine, University of Fukui, 23-3, Matsuoka Shimoaizuki, Eiheiji-cho, Yoshida-gun, Fukui, 910-1193 Japan
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Liu Y, Hao H, Guo X, Xu J, Kang L, Zheng G, Zhao H. Rare pancreatic carcinosarcoma in a patient with medical history of esophageal cancer: A case report and literature review. Medicine (Baltimore) 2019; 98:e15238. [PMID: 31008956 PMCID: PMC6494381 DOI: 10.1097/md.0000000000015238] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/14/2022] Open
Abstract
RATIONALE Pancreatic carcinosarcoma (PCS) is a very rare pancreatic cancer with an extremely poor prognosis. Interestingly, PCS can coexist with other metachronous malignant cancers. Here we report a case of PCS combined with esophageal cancer (EC). PATIENT CONCERNS The patient was a 66-year-old man who presented with abdominal pain and progressive nausea. He had undergone esophagectomy for EC 5 years previously. DIAGNOSIS Both EC and PCS were confirmed via postoperative pathological diagnosis. INTERVENTIONS Owing to the patient's previous esophagectomy for EC, pancreaticoduodenectomy for the PCS could not be performed. Instead, he underwent cholecystectomy with bile duct-jejunum Roux-en-Y anastomosis and radioactive seed implantation. OUTCOMES The patient is still alive for >1 year. LESSONS To our knowledge, this is the first report of PCS combined with EC and thus of metachronous multiple primary carcinoma. A detailed literature review of the clinical and histologic features of PCS reveals important information about the epidemiology and biology of this rare disease.
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Affiliation(s)
- Yang Liu
- Department of Pathology, Hebei General Hospital
| | - Han Hao
- Department of Pharmacology, Hebei Medical University
| | - Xiaowan Guo
- Department of Image, Hebei General Hospital, Shijiazhuang
| | - Jieping Xu
- Tianjin Hexi Women & Children Healthcare Center, Tianjin, China
| | - Lin Kang
- Department of Pathology, Hebei General Hospital
| | - Guona Zheng
- Department of Pathology, Hebei General Hospital
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Youngwirth LM, Freischlag K, Nussbaum DP, Benrashid E, Blazer DG. Primary sarcomas of the pancreas: A review of 253 patients from the National Cancer Data Base. Surg Oncol 2018; 27:676-680. [PMID: 30449492 DOI: 10.1016/j.suronc.2018.08.006] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/23/2018] [Revised: 07/16/2018] [Accepted: 08/27/2018] [Indexed: 10/28/2022]
Abstract
INTRODUCTION Primary sarcomas of the pancreas are rare, and the limited data regarding their presentation, oncologic profile, and survival have been derived from small case series. METHODS The National Cancer Data Base (1998-2012) was queried for patients with primary sarcomas of the pancreas. Demographic and clinical features at the time of diagnosis were evaluated for all patients. Subjects who underwent surgical resection were identified, and logistic regression was used to identify variables associated with resection. A Cox proportional hazards model was developed to identify factors associated with survival. RESULTS In total, 253 patients were identified. The mean age at diagnosis was 63 years, with tumors occurring more frequently in women (57.3%) and those of white race (79.8%). Tumors in the head of the pancreas were most common (63.3%). The mean size was 7.5 cm. Only 100 patients (39.5%) underwent resection, with younger age (OR = 0.763, p = 0.04) and smaller tumor size (OR = 0.978, p < 0.01) associated with resection. Chemotherapy and radiation therapy use were similar in patients who underwent resection and those who did not. Patients who underwent resection had a median survival of 17 months, compared to 6 months for patients who were not resected (p < 0.01). Following adjustment, only older age (HR 1.257, p = 0.03) and higher tumor grade (HR 1.997, p = 0.01) were associated with an increased risk of death in resected patients. CONCLUSIONS Primary pancreatic sarcomas are rare and the majority of patients do not undergo resection; thus, little is known about their oncologic profile or outcomes following pancreatectomy. Patients who undergo resection have markedly improved survival; older age and higher tumor grade are associated with decreased survival.
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Xie Y, Xiang Y, Zhang D, Yao X, Sheng J, Yang Y, Zhang X. Sarcomatoid carcinoma of the pancreas: A case report and review of the literature. Mol Med Rep 2018; 18:4716-4724. [PMID: 30221744 DOI: 10.3892/mmr.2018.9489] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/10/2018] [Accepted: 09/07/2018] [Indexed: 12/15/2022] Open
Abstract
Sarcomatoid carcinoma (SC) is an extremely rare and complicated malignant neoplasm that consists of both malignant epithelial components and atypical spindle cells that express an epithelial phenotype. The presents study reported a case of SC of the pancreas (SCP), along with a brief review of the literature. A 63‑year‑old man was admitted to The Second Hospital of Jilin University hospital with complaints of epigastralgia and jaundice of one month in duration. Based on preoperative laboratory blood tests and radiography, a mass at the distal common bile duct was suspected. Intraoperative examination discovered a 2.5x2x1.8‑cm mass in the pancreatic head, with invasion of the distal bile duct. Pancreaticoduodectomy was performed. Histopathology and immunohistochemistry of the specimen confirmed the diagnosis of SCP. The patient succumbed 18 months after surgery due to multiple hepatic metastases.
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Affiliation(s)
- Yingjun Xie
- Department of Hepatobiliary and Pancreatic Surgery, The Second Hospital of Jilin University, Changchun, Jilin 130041, P.R. China
| | - Yien Xiang
- Department of Hepatobiliary and Pancreatic Surgery, The Second Hospital of Jilin University, Changchun, Jilin 130041, P.R. China
| | - Dan Zhang
- Department of Hepatobiliary and Pancreatic Surgery, The Second Hospital of Jilin University, Changchun, Jilin 130041, P.R. China
| | - Xiaoxiao Yao
- Department of Hepatobiliary and Pancreatic Surgery, The Second Hospital of Jilin University, Changchun, Jilin 130041, P.R. China
| | - Jiyao Sheng
- Department of Hepatobiliary and Pancreatic Surgery, The Second Hospital of Jilin University, Changchun, Jilin 130041, P.R. China
| | - Yongsheng Yang
- Department of Hepatobiliary and Pancreatic Surgery, The Second Hospital of Jilin University, Changchun, Jilin 130041, P.R. China
| | - Xuewen Zhang
- Department of Hepatobiliary and Pancreatic Surgery, The Second Hospital of Jilin University, Changchun, Jilin 130041, P.R. China
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Abstract
Carcinosarcomas are rare biphasic neoplasms with distinct malignant epithelial and mesenchymal components. Most commonly, carcinosarcomas arise in the uterus as malignant mixed müllerian tumors, but also infrequently appear in other organs such as the ovaries and breast, the prostate and urinary tract, the lungs, or in the gastrointestinal system, among others. Pancreatic carcinosarcomas are exceedingly rare; only a few cases are reported in the English literature. Their pathogenesis remains to be fully clarified. We present here the case of a pancreatic carcinosarcoma with evidence for monoclonality via determination of Kras mutational status after microdissection and suggest a common origin of the 2 tumor components. Comprehensive review of the available literature allows the conclusion that most pancreatic carcinosarcomas appear to be of monoclonal origin and seem to have arisen from a carcinoma via metaplastic transformation of 1 part or subclone of the tumor, probably by epithelial-mesenchymal transition. All reported patients were treated with surgery. Adjuvant therapy, if administered, consisted predominantly of gemcitabine. Prognosis for this neoplasm occurs to be similar or even worse compared with classic pancreatic ductal adenocarcinoma. Despite the lack of evidence-based recommendations for its treatment, resection should be performed, if possible.
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Mszyco S, Teng L, Annunziata J, Hartman MS. Pancreatic Carcinosarcoma: A Case Report Highlighting Computed Tomography Characteristics. Curr Probl Diagn Radiol 2017; 46:342-345. [PMID: 28318763 DOI: 10.1067/j.cpradiol.2017.01.007] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/01/2016] [Revised: 01/18/2017] [Accepted: 01/20/2017] [Indexed: 01/23/2023]
Abstract
Primary pancreatic carcinosarcoma is a rare and malignant neoplasm arising within the pancreas. With fewer than a dozen cases ever reported, the recognition and treatment of this rare phenomenon can be difficult. We describe the case of an 85-year-old man who presented with vague right upper quadrant and epigastric abdominal pain and was found to have a pancreatic mass on ultrasound and computed tomography. The patient underwent open pancreaticoduodenectomy and was found to have pancreatic carcinosarcoma on microscopic evaluation. Although specific radiologic diagnosis of this entity is not possible, bringing the possibility of primary pancreatic carcinosarcoma to the ordering clinician׳s attention has the potential to hasten treatment and improve patient outcomes. We review the current literature on this rare type of neoplasia, considering histopathologic and clinical features. This case highlights the importance of radiologist awareness of this rare neoplasm and to consider carcinosarcoma in the differential when presented with a mixed solid and cystic pancreatic mass.
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Affiliation(s)
- Steven Mszyco
- Department of Radiology, Dartmouth-Hitchcok Medical Center, Lebanon, NH.
| | - Leslie Teng
- Department of Radiology, Allegheny Health Network, Pittsburgh, PA
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Li BQ, Liu QF, Chang XY, Hu Y, Chen J, Guo JC. Pancreatic carcinosarcoma mimics malignant intraductal papillary mucinous neoplasm: A rare case report and literature review. Medicine (Baltimore) 2017; 96:e6961. [PMID: 28591030 PMCID: PMC5466208 DOI: 10.1097/md.0000000000006961] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/22/2022] Open
Abstract
RATIONALE Carcinosarcoma, an extremely rare pancreatic primary tumor, is characterized by coexistence of both carcinomatous and sarcomatous components. Due to its rarity, the clinical manifestation and imaging features have not been recognized. An accurate diagnostic method has not been available and a widely accepted guidelines instructing treatment has not been established. PATIENT CONCERNS We present an uncommon case of pancreatic carcinosarcoma (PCS) which has been preoperatively diagnosed as pancreatic malignant intraductal papillary mucinous neoplasm. A radical resection, including total pancreatectomy (TP) and splenectomy, was performed. DIAGNOSIS The diagnosis of PCS was confirmed by postoperative pathology. INTERVENTIONS A radical resection, including TP and splenectomy, was performed. The patient was followed up by abdominal contrast-enhanced computed tomography scan and blood tumor marker examination. OUTCOMES The patient is still alive and self-sufficient 7 months after the surgery. No evidence of tumor recurrence is found during follow-up. LESSONS Although, until recently, there are no widely accepted guidelines instructing treatment for PCS, a radical resection is still a possible way. All the pancreatic neoplastic patients with high surgical risk should be transferred to a specialized high-volume pancreatic center to get precise preoperative evaluation, fine operation technique, and careful postoperative management.
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Affiliation(s)
| | | | - Xiao-Yan Chang
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Ya Hu
- Department of General Surgery
| | - Jie Chen
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
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Jia Z, Zhang K, Huang R, Zhou X, Jiang L. Pancreatic carcinosarcoma with rare long-term survival: Case report and review of the literature. Medicine (Baltimore) 2017; 96:e5966. [PMID: 28121946 PMCID: PMC5287970 DOI: 10.1097/md.0000000000005966] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/30/2022] Open
Abstract
PATIENT CONCERNS We report a rare case of pancreatic carcinosarcoma involving a 44-year-old woman. The patient complained of discomfort associated with the upper abdomen and jaundice of skin and sclera for 1 week. DIAGNOSES After hospitalization, relevant examinations were completed. The disease was diagnosed as carcinoma of the pancreatic head. INTERVENTIONS Whipple procedure was conducted in May 2013. Intraoperative exploration indicated 2 components of the tumor: a fish-shaped gray matter and a hard structure similar to cancellous bone. Histopathological examination showed adenocarcinoma and osteosarcoma. After surgery, the patient received 8 cycles of chemotherapy with gemcitabine and raltitrexed. OUTCOMES Previous studies indicated poor prognosis for pancreatic carcinosarcoma. However, our patient survived for 31 months with no recurrence till date. LESSONS SUBSECTIONS Coexistence of pancreatic adenocarcinoma and osteosarcoma is very rare. Our case was also an exception in manifesting longer survival than expected.
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Affiliation(s)
- Zhe Jia
- Department of General Surgery
| | | | | | - XinGang Zhou
- Department of Pathology, Beijing Ditan Hospital, Capital Medical University, Chaoyang District, Beijing, P.R. China
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Bai Q, Zhang X, Zhu X, Wang L, Huang D, Cai X, Zhou X, Wang J, Sheng W. Pancreatic carcinosarcoma with the same KRAS gene mutation in both carcinomatous and sarcomatous components: molecular evidence for monoclonal origin of the tumour. Histopathology 2016; 69:393-405. [PMID: 27307095 DOI: 10.1111/his.12975] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/13/2015] [Accepted: 03/25/2016] [Indexed: 12/12/2022]
Abstract
AIMS To better understand the histogenesis, prognosis and feasible treatment of pancreatic carcinosarcoma, a rare type of neoplasia. METHODS AND RESULTS We investigated eight additional cases of pancreatic carcinosarcoma at a single institution, including the clinicopathological, immunohistochemical, and KRAS mutation characteristics. We have also reviewed the current literature on this rare type of neoplasia, and summarized the clinicopathological features and feasible treatments. As a result, concordant strong nuclear immunoreactivity for P53 protein and the same type of KRAS gene mutation, c.35G>A (p.G12D) or c.35G>T (p.G12V), were showed in both carcinomatous and sarcomatous components in five of eight cases. Furthermore, we found that the patients treated with surgery plus postoperative chemotherapy had longer survival than those treated with surgery only (P = 0.034 and P = 0.131 for overall survival and disease-free survival, respectively), although Cox regression multivariate analysis indicated that it was not an independent predictor. In addition, we found KRAS mutant allele-specific imbalance in four of our five cases of pancreatic carcinosarcoma, which was associated with advanced disease and a worse prognosis. CONCLUSIONS This is the largest panel of cases of pancreatic carcinosarcoma studied so far, including clinicopathological, immunohistochemical and molecular cytogenetic features. Our findings indicate that the tumour could have been of monoclonal origin, and that the sarcomatous components might have arisen from metaplastic transformation of the carcinomatous components. Our results also suggest that surgery plus POC including gemcitabine may be a good choice for patients with pancreatic carcinosarcoma.
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Affiliation(s)
- Qianming Bai
- Department of Pathology, Fudan University Shanghai Cancer Centre, Shanghai, China.,Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, China
| | - Xin Zhang
- Department of Pathology, Fudan University Zhongshan Hospital, Shanghai, China
| | - Xiaoli Zhu
- Department of Pathology, Fudan University Shanghai Cancer Centre, Shanghai, China.,Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, China
| | - Lei Wang
- Department of Pathology, Fudan University Shanghai Cancer Centre, Shanghai, China.,Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, China
| | - Dan Huang
- Department of Pathology, Fudan University Shanghai Cancer Centre, Shanghai, China.,Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, China
| | - Xu Cai
- Department of Pathology, Fudan University Shanghai Cancer Centre, Shanghai, China.,Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, China
| | - Xiaoyan Zhou
- Department of Pathology, Fudan University Shanghai Cancer Centre, Shanghai, China.,Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, China
| | - Jian Wang
- Department of Pathology, Fudan University Shanghai Cancer Centre, Shanghai, China.,Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, China
| | - Weiqi Sheng
- Department of Pathology, Fudan University Shanghai Cancer Centre, Shanghai, China.,Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, China
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15
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Shi HY, Xie J, Miao F. Pancreatic carcinosarcoma: First literature report on computed tomography imaging. World J Gastroenterol 2015; 21:1357-1361. [PMID: 25632213 PMCID: PMC4306184 DOI: 10.3748/wjg.v21.i4.1357] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/05/2014] [Revised: 08/06/2014] [Accepted: 09/30/2014] [Indexed: 02/06/2023] Open
Abstract
Carcinosarcoma of the pancreas is an extremely rare tumor and has a dismal prognosis. To the best of our knowledge, the histopathological features of the lesion have been illustrated in the literature but to date no reported cases have been documented on imaging characteristics. We report a female case of pancreatic carcinosarcoma presenting as a mucinous cystadenoma on computed tomography (CT). We also summarize the CT characteristics according to the appended CT images in the reported cases. This is the first report of CT features of pancreatic carcinosarcoma in the English literature.
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16
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Santhosh S, Mittal BR, Rana SS, Srinivasan R, Bhattacharya A, Das A, Bhasin D. Metabolic signatures of malignant and non-malignant mass-forming lesions in the periampulla and pancreas in FDG PET/CT scan: an atlas with pathologic correlation. ACTA ACUST UNITED AC 2014; 40:1285-315. [DOI: 10.1007/s00261-014-0266-y] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
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17
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Kim HS, Kim JI, Jeong M, Seo JH, Kim IK, Cheung DY, Kim TJ, Kang CS. Pancreatic adenocarcinosarcoma of monoclonal origin: A case report. World J Gastroenterol 2014; 20:12682-12686. [PMID: 25253976 PMCID: PMC4168109 DOI: 10.3748/wjg.v20.i35.12682] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/25/2014] [Revised: 04/30/2014] [Accepted: 07/25/2014] [Indexed: 02/06/2023] Open
Abstract
Adenocarcinosarcoma, a neoplasm containing both carcinomatous and sarcomatous components, is a rare form of a cancer and the pathophysiology is currently poorly understood. Moreover, definitive treatment guidelines for this disease have not yet been established. Pancreatic adenocarcinosarcoma is even more rare and the prognosis is fatal. Here, we report a case of a 77-year-old male with pancreatic adenocarcinosarcoma and metastasis to the liver. The patient presented at our hospital with uncontrolled glucose levels and diabetes mellitus. The patient’s laboratory findings were unremarkable with the exception of elevated carbohydrate antigen 19-9 levels. Biopsies of the tumors in the pancreas and the liver revealed two types of tumors: pancreatic adenocarcinoma and a poorly differentiated sarcoma. To determine if KRAS mutations were present, we performed a peptide nucleic acid (PNA) clamp PCR-based assay. DNA sequencing by PNA clamp PCR identified a point mutation in codon 12 of exon 2 within KRAS from both tumor types. Because the KRAS mutation is observed in both tumor components, our findings support a monoclonal tumor origin followed by subsequent divergent differentiation into the sarcomatous and carcinomatous tumor populations. After we considered the patient’s status and the late stage of tumor detection, gemcitabine chemotherapy was administered.
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18
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Yao J, Qian JJ, Zhu CR, Bai DS, Miao Y. Laparoscopic left pancreatectomy for pancreatic sarcomatoid carcinoma: A case report and review of the literature. Oncol Lett 2013; 6:568-570. [PMID: 24137372 PMCID: PMC3789049 DOI: 10.3892/ol.2013.1411] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/14/2013] [Accepted: 05/24/2013] [Indexed: 02/06/2023] Open
Abstract
Sarcomatoid carcinoma of the pancreas is extremely rare. The current report presents a case of carcinosarcoma of the pancreas in a 48-year-old male. Pre-operative computed tomography scans revealed a large complex cystic and solid mass in the tail of the pancreas; the patient underwent a laparoscopic spleen-preserving left pancreatectomy. The tumor was shown to be made of cystic and solid components, with a grossly grey/ white appearance. A histological evaluation of the tumor revealed two elements separated from each other, one component was a pancreatic ductal adenocarcinoma and the other component exhibited a sarcomatous growth pattern, composed of spindle cells and multinucleated giant cells. Immunohistochemically, the epithelial area was positive for cytokeratin (CK) and negative for vimentin, while the sarcomatoid area was negative for CK and positive for vimentin. These observations confirmed a diagnosis of pancreatic carcinosarcoma. Although the patient was treated by gemcitabine following surgery, the outcome was extremely poor and the patient succumbed to sarcomatoid carcinoma three months after the treatment.
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Affiliation(s)
- Jie Yao
- Departments of Hepatobiliary and Pancreatic Surgery, The First Affiliated Hospital of Yangzhou University, Yangzhou, Jiangsu 225001, P.R. China
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